Neurological Emergencies

Status Epilepticus

Causes of Seizures

Vascular (SAH, venous sinus thrombosis, hypertensive enceph)

InfectiousTraumaticAutoimmune (SLE)Metabolic/Toxic (hypo-/hypernatremia,

hypo-/hyperglycemia, alcohol intoxication/withdrawal, INH)

IdiopathicNeoplasticStructural/congenital

Status Epilepticus

• Continuous tonic-clonic seizure activity greater than 15 minute duration, or recurrent GTCs with no regain in consciousness

• A medical emergency!!!– Adverse consequences can include hypoxia,

hypotension, acidosis and hyperthermia– Know the recommended sequential protocol for

treatment with benzodiazepines, phenytoin, and barbiturates.

• Goal: stop seizures as soon as possible

Pathophysiology

• Early compensation meets increased CNS metabolic needs (SBP, CBF ↑↑)

• Failure at 40-60 minutes, (SBP, CBF ↓↓) • CNS tissue necrosis, adverse sequelae• Glutamate toxic mediator• CNS necrosis even if systemic

complications fully mitigated• HPN, fever, rhabdomyolysis, hypercarbia,

hypoxia, infection

Status Epilepticus: SE Working Group (Consensus Document)

• Management must simultaneously address:– Stabilization: ABCs– Diagnostic testing including rapid glucose– Pharmacologic interventions

• Drug therapy– Diazepam 5 mg IV q 5 min x 4 doses– Phenytoin 20 mg/kg at 25-50 mg/min – Repeat phenytoin 5 mg/kg – Phenobarbital 20 mg/kg at 100 mg/min – Valproic acid 20 mg/kg

Epilepsy Foundation of America. JAMA 1993;270:854-859Epilepsy Foundation of America. JAMA 1993;270:854-859

CNS Infections

Cardinal Manifestations of CNS Infections

• Fever

• Headache

• Alteration of Sensorium Seizures

• Meningismus Other focal neurologic signs

Main CNS Infection SyndromesAcute Meningitis (Days) Bacterial meningitis

Viral meningitis

Subacute to Chronic meningitis (Days to Weeks)

Tuberculous meningitis

Cryptococcal meningitis

Partially treated bacterial meningitis

Space-Occupying Lesions Brain/Spinal Abscess

Subdural empyema

Cysticercosis

Chronic CNS Infection (Months to Years)

Neurosyphilis

Prion diseases

Diagnostics in CNS Infection

• CSF analysis– Lumbar puncture– Cisternal puncture– Ventricular tap– Q/Q, GS/CS,AFB, CALAS

• Neuroimaging– CT scan– MRI

CSF Profiles

WBCs/mm3

Cell Type Protein(mg/dl)

Glucose(mg/dl)

OpeningPressure(cm H2O)

Normal ≤5 Lymphos and monos

only

15 – 45 45 – 80 8 – 18

Bacterialmeningitis

5 – 10,000

PMNs ↑ ↓ ↑

Viralmeningitis

5 – 1,000

Lymphos ↑ N N, occ’l ↑

Tuberculousmeningitis

5 – 500 Lymphos ↑ ↓ ↑

Cryptococcalmeningitis

5 – 100 Lymphos ↑ N, occ’l ↓ ↑

Treatment

• Bacterial Meningitis– Antibiotics

• TB Meningitis– Anti-Koch’s– VPS for hydrocephalus– Steroids for arteritis

Empiric Antibiotic Treatmentfor Bacterial Meningitis

Risk Group Etiologies Antibiotic Coverage

Neonates (< 1 mo) Group B or Group D streptococciGram-negative rods (E. coli)Listeria monocytogenes

Ampicillin 50 mg/kg IV q 6-8Cefotaxime 50 mg/kg IV q 8

Children(3 mos - 7 y.o.)

Haemophilus influenzaeStreptococcus pneumoniaeNeiserria meningitidis

Ceftriaxone 50 mg/kg IV q 12 h

Young adults(7 - 50 y.o.)

S. pneumoniaeN. meningitidis

Vancomycin 1 g IV q 12 hCeftriaxone 2 g IV q 12 h

Adults > 50 y.o.alcoholics, Pts with debilitating medical condition

S. pneumoniaeL. monocytogenesGram-negative rods

Ampicillin 2 gm IV q 4 hCeftriaxone 2 g IV q 12 h

Patients w postneurosurgical procedure or head trauma

S. aureusS. pneumoniaeGram-negative rods

Vancomycin 1 gm IV q 12Ceftazidime 2 gm IV q 8 h

Acute Weakness Resulting from Lower Motor Neuron Lesions

Causes of Acute Weakness in Previously Healthy Patients

• Anterior Horn Cell– Poliomyelitis

• Nerve– Acute Inflammatory Demyelinating Polyneuropathy (GBS)– Diphtheria– Tick paralysis– Heavy metal intoxication

• Neuromuscular– Myasthenia gravis– Lambert Eaton syndrome– Botulism– Organophosphate poisoning

• Muscle– Polymyositis– Periodic paralysis (hypokalemia, thyrotoxic)– Toxic myopathy– Rhabdomyolysis– Malignant hyperthermia

Guillain-Barre Syndrome

Peripheral Nerves

Acute Inflammatory Demyelinating Polyneuropathy (AIDP,GBS)

• History– Antecedent infection or immunization– Progressive relatively symmetrical weakness– Bulbar weakness and ataxia or respiratory muscle

dysfunction may predominate

• Examination– Hypo-/areflexia– Motor weakness– Facial diplegia– Minimal objective sensory deficits– Normal mentation

• Laboratory– Lumbar tap and CSF analysis with elevated

protein and less than 10 WBC (albumino-cytologic dissociation)

– Electromyography with prolonged F waves, decreased nerve conduction velocity or conduction block

• Management– Consider plasmapheresis or IVIG– Evaluate respiratory function, ventilate if

necessary– Monitor cardiac rhythm and hypotension

Acute Inflammatory Demyelinating Polyneuropathy

Acute Weakness Resulting from Neuromuscular Junction Disorder

(Myasthenia Gravis)

Clinical Manifestations

• Fluctuating weakness and muscular fatigue, affecting ocular, bulbar, and peripheral (skeletal) muscles

• 50-60% will present with diplopia and ptosis as early primary features

• Isolated extraocular and palpebral muscle weakness may be the only initial manifestation in some patients (Ocular Myasthenia Gravis).

• 85% to 90% of patients presenting with ocular symptoms will eventually develop more generalized weakness

•With generalized disease, extremity weakness, usually involving the proximal upper and lower extremities and the extensor muscles, is common and typically worsens with exertion

•Most serious complication is respiratory muscle weakness, which may progress to hypoventilation and respiratory failure

Classification

I : Ocular myasthenia (14% stay at this stage)

IIA: Mild generalized myasthenia with ocular signs

IIB: Moderately severe generalized myasthenia with mild bulbar and ocular involvement

III: Acute severe, with bulbar and respiratory complications (Myasthenic Crisis)

IV: Late severe, developing from other groups within 2 years

Diagnostic Methods

• Tensilon (Edrophonium) test:– Sensitivity is 90%, seen also in other

neuromuscular diseases– WOF hypotension, syncope, respiratory failure

• AChR antibodies– Sensitivity 90%, specificity 100%

• Electrodiagnostic– RNS Sensitivity 50%– SFEMG Sensitivity 90% (also in other

diseases)

Treatment of MG

• Acetylcholinesterase inhibitors Reversible binding to AchE, accumulation of

Ach at post-synaptic membrane Pyridostigmine (Mestinon): Onset 15-30m,

Peak effect 1-2h; Wearing off 3-4 H WOF: Cholinergic crisis

• IVIG

• Plasmapharesis

Steroids in MG

• Sustained improvement appears in most patients within 2 weeks, with improvement in 90% of patients within 3 weeks

• Mild exacerbation within 1 to 17 days after starting glucocorticoids (most commonly starting on day 5), but lasts only 4 days on average

• Induces effective remission in up to 80% of patients

Thymectomy in MG

• Has been incompletely studied and most patients in trials were also treated with additional immunosuppressive therapies

• Lasting improvement following thymectomy is delayed for 6 to 12 months and may not appear for several years

• Up to 60% to 70% of patients with onset before 40 years of age and no thymoma may improve after surgery